Spiegel J E, Willenbucher R F
Department of Internal Medicine, University of California, San Francisco, UCSF-Mount Zion Medical Center, 94114-2518, USA.
JPEN J Parenter Enteral Nutr. 1999 May-Jun;23(3):169-72. doi: 10.1177/0148607199023003169.
A 32-year-old man with active Crohn's disease and recurrent small bowel strictures underwent abdominal surgery and was subsequently given total parenteral nutrition (TPN). Severe cholestasis developed and copper was removed from the TPN. Although serum ceruloplasmin levels were within normal limits, 8 weeks after copper removal, he developed pancytopenia. Serum copper levels were severely depressed. Bone marrow biopsy was consistent with copper deficiency; cytoplasmic vacuolization of both myeloid and erythroid precursors, megaloblastic erthropoiesis, and marked hypocellularity were observed. IV replacement with copper sulfate resulted in improvement in the patient's anemia, neutropenia, and thrombocytopenia, but the patient died suddenly from cardiac tamponade. Postmortem examination revealed fibrinous and hemorrhagic pericarditis. Despite the rare occurrence of overt copper deficiency, this case emphasizes the need to recognize copper deficiency as an important etiology of iron-resistant anemia in patients receiving TPN. Furthermore, the relative rapidity with which our patient developed pancytopenia suggests that, in view of the established recommendation that copper be removed from TPN in cholestatic conditions, serum copper levels must be measured periodically.
一名32岁患有活动性克罗恩病且反复出现小肠狭窄的男子接受了腹部手术,随后接受了全胃肠外营养(TPN)。出现了严重的胆汁淤积,遂从TPN中去除了铜。尽管血清铜蓝蛋白水平在正常范围内,但在去除铜8周后,他出现了全血细胞减少。血清铜水平严重降低。骨髓活检符合铜缺乏表现;观察到髓系和红系前体细胞的细胞质空泡化、巨幼红细胞生成以及明显的细胞减少。静脉注射硫酸铜后,患者的贫血、中性粒细胞减少和血小板减少有所改善,但患者突然死于心脏压塞。尸检显示为纤维蛋白性和出血性心包炎。尽管明显的铜缺乏很少见,但该病例强调了需要认识到铜缺乏是接受TPN患者铁抵抗性贫血的重要病因。此外,我们的患者全血细胞减少发展相对迅速,这表明鉴于在胆汁淤积情况下从TPN中去除铜的既定建议,必须定期测量血清铜水平。